Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case.
Ectopic adrenocortical neoplasms arising in the nervous system are very rare. We encountered an intradural, extramedullary case of an adrenocortical neoplasm of indeterminate malignant potential affecting a spinal nerve root in the distal lumbar region of a 5-month-old girl. The lesion recurred 6 months after the initial gross total resection. The tumor in both resections had increased mitotic activity (5/10 high power fields) and MIB-1 labeling indices of 23% and 33% at initial resection and recurrence, respectively. Both tumors demonstrated gains of chromosomes 5 and 12 by interphase cytogenetics, whereas insulin growth factor 2 was identified in the recurrent tumor by immunohistochemistry. This report demonstrates that ectopic adrenocortical tumors in the nervous system may exhibit clinicopathologic and cytogenetic features suggestive of adrenocortical carcinoma.
Rodriguez
Fausto J
FJ
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA. rodriguez.fausto@mayo.edu
Scheithauer
Bernd W
BW
Erickson
Lori A
LA
Jenkins
Robert B
RB
Giannini
Caterina
C
D000306:Adrenal Cortex Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D008279:Magnetic Resonance Imaging; D046529:Microscopy, Electron, Transmission; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D013126:Spinal Nerve Roots / Q000378:metabolism / Q000473:pathology*